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Adult cystic fibrosis centre

Patient information

We run three cystic fibrosis (CF) outpatient clinics a week on Mondays, Tuesdays and Friday afternoons along with a monthly CF-related diabetes clinic on the last Friday of every month. The clinics are all held in outpatients west at Royal Brompton Hospital.

We also have a CF gastroenterology clinic which is held in Chelsea and Westminster hospital every fourth Friday of the month.

Additionally, we run a day-case unit where patients can be reviewed by the medical team if necessary (by appointment only).

Please note that all clinics mentioned above are closed during UK bank holidays (England and Wales).

CF Trust guidelines

We follow the CF Trust's guidelines on cross-infection for both inpatients and outpatients. Therefore, the clinics are segregated and, on arrival, patients go into separate consulting rooms. So as to prevent cross-infection, patients growing different bugs in their sputum are seen on different days.

Appointments

Clinics operate on an appointment system. To minimise waiting times patients will be seen in appointment order rather than arrival time.

When you arrive at clinic, please book in with reception. You will then be allocated a clinic room where you will be weighed. Other members of the CF team, including the dietitian, the physiotherapist, the CF nurse specialist and the doctor, will visit to assess and talk to you. You will have your lung function completed (blowing or spirometry tests), your oxygen levels checked, a sputum sample or cough swab taken, and you may be asked to go for an X-ray or blood tests, if these are required.

When you have finished in clinic, please hand your clinic slip to the reception desk where they will be able to book a follow-up appointment for you.

If you are unable to attend a clinic, please call the appointments line on 020 7351 8011 in advance so you can rebook the appointment.

If you are feeling unwell, and feel you need to be seen by the team earlier than your next appointment, please call the CF nurse specialists on 020 7351 8065. They will be able to advise you on how to proceed. If you happen to feel unwell overnight or at a weekend and feel unable to wait until the next working day, you should phone the main hospital switchboard on 020 7352 8121 and ask to talk to the on-call respiratory registrar for adults.

Transition clinics

Transition clinics are held for patients wishing to transfer their care to the adult clinic. The aim of these clinics is to make the transition from the paediatric to the adult service easier for both the patient and family. These clinics are run jointly with the adult and paediatric teams from Royal Brompton and Great Ormond Street. However, due to geography or choice, we also have patients transition to the adult service from other paediatric CF clinics in the London area.

Having intravenous (IV) antibiotics at home (home IVs) is now a common part of cystic fibrosis treatment.

A decision to start IVs at home is influenced by your condition at the time and if the team feel it is an appropriate method for your treatment. The medical team must be confident that this is the best option for you and that you will be able to take on this extra responsibility as well as continue with your usual day to day treatment. It is also important to consider how much help and support you will have at home. IVs at home may not be appropriate for every exacerbation and a decision will need to be made each time. Home IVs do not suit everyone and for some people hospital is the preferred option.

Advantages

Home IVs can reduce time in hospital.

Dose administration times can be adjusted to suit your lifestyle.

Home IVs may allow your daily routine to continue.

Home IVs may avoid time off work/college.

Home IVs may reduce child care issues.

You can continue to eat the foods you like and cook at home.

Disadvantages

There is less visible support from the medical team.

There is less intensive physio input and nutritional support.

Monitoring your response to treatment will be more difficult.

You may need to make visits to hospital for levels of if your IV access fails.

Emergency help is not available to hand.

No guaranteed bed rest.

What happens next?

If you and the medical team decide that home IVs are the appropriate choice then you will be assessed either on Lind ward day unit or, if already an inpatient, then on the ward prior to discharge.

If you do not have a portacath, a qualified nurse will assess your veins and discuss whether a long line or venflon is more suitable. The qualified nurse will teach you to draw up drugs, safely administer IV antibiotics and care for your IV access is carried out by a suitable nurse. The learning process is tailored to each person. There is no time limit and you will be encouraged to pace yourself and ask as many questions as you need. No question is too big or too small so use the time to ask anything at all. You will be assessed on each occasion even if you have done home IVs before. This is mainly to refresh your memory and give an opportunity for you to ask questions as procedures may change from time to time.

After you have been assessed

You will be monitored for one hour after your first dose of treatment. This is a must on every occasion as adverse reactions and side effects can occur even if you have had the medication on numerous occasions before.Not staying for the required hour will affect your opportunity to continue doing home IVs in the future.

Our pharmacy department will provide you with 3 doses of your treatment and the rest will be delivered to your home, usually the next day by a company called Willow. They will phone you, usually on the day you commence treatment to agree a suitable delivery time. You will also be provided with a sharps bin and fridge to store your treatments if you require one.

Monitoring your response to treatment

If you are commenced on aminoglycosides (eg:Tobramycin) you will need to have blood levels done to help us monitor your response to these treatments. It is extremely important to have checks as these treatments can be toxic. These levels have to be done and cannot be missed. If blood levels are missed this may affect you being considered to do further IVs at home in the future

Depending on the day you start treatment you may be able to do your own blood levels at home. Levels for Tobramycin are taken on day 3 and day 7 before your dose. They can be done by pricking your finger with a fine needle, putting some blood into a special tube and posting it back to us in a prepaid box. Other options are to come back to the hospital to have them done if they are due on a weekend day or in special circumstances by your GP.

The results of your levels will be checked by the nurse specialists and you will only be contacted if there are any abnormal results.

Home IVs commenced on Mondays, Tuesdays or Wednesdays mean you can post your levels back to the Brompton. If you start IVs on a Thursday or Friday you will need to come to Foulis ward for your levels due to weekend post. You will be seen by one of the nurse specialists before you leave who will explain all of this in more detail.

Support available

There is on-going support from the team whilst doing your treatment at home. The CF medical team and nurse specialists are available Monday to Friday from 9am-5pm. There is an on-call service at weekends.

You will receive a call from one of the nurse specialists on a Tuesday afternoon during the telephone clinic to see how you are doing.

You will be seen in clinic or on Lind ward at the end of your course to see how you are doing.

You will be given a request slip to take downstairs to the administrators to book a follow up appointment.

You should ensure you have a date to return before you leave to go home.

You must attend your follow up appointment. Not coming may also affect your opportunity to do any future IVs at home. We realise that we have mentioned this fact a few times throughout this information page but it is in your best so you have the safest and most effective treatment possible.

It may be possible to continue with work or education whilst having home IVs and this can be discussed with the medical team when you commence treatment. It is worth mentioning that there is a school team that can help with your education whilst an inpatient too. It is important to stress that thorough physiotherapy, good nutrition and adequate rest are all essential towards achieving the best outcome from your treatment.

What is an annual review?

Your CF annual review is a bit like an annual car service - everything gets checked!

Clinics are busy and although you attend regularly it can be easy to overlook an aspect of your care in this environment. To prevent this we arrange an annual review. This is a comprehensive multidisciplinary overview that includes tests, investigations and assessments. The results provide an update on your health and help in the establishment of your management plan for the following year.

You will need to visit Royal Brompton Hospital twice for your annual review. This is so that results of tests taken during the first visit are available for your second visit.

First visit

The first visit is a “fact finding mission” and involves investigations such as lung function, blood tests (including vitamin A, D and E levels), chest X-ray, oral glucose tolerance test (for people who do not have CF-related diabetes), a sputum specimen and a bone density scan (if required).

There are also assessments by the dietitian, physiotherapist and clinical nurse specialist.

The physiotherapist will talk with you about your airway clearance technique, exercise, posture, any leakage of wind/urine when coughing or laughing, and sometimes a six-minute walk test.

The CF nurse specialist will discuss your health during the previous year, review your medication, allergies and medication sensitivities and hospital admission history. You will be asked about employment, education/studies, family support and living arrangements, contraception and fertility status so that we can provide you with any information you need or refer you to the appropriate person. The roles of the welfare rights adviser and the psychology service will be explained and a referral can be made if required.

Before you are seen by the pharmacist, they will review your medication history from your most recent clinic or discharge letter, and your GP medication record. The pharmacist will then review and discuss each of your medicines with you at the review, and any issues or problems you may be experiencing with your medicines will also be discussed. The pharmacist will also ask about any allergies you have, and answer any questions you have about your medicines.

Second visit

The second visit is an important appointment with a CF consultant. This appointment is your opportunity to discuss your management plan for the following year based on the results of your investigations and assessments - your chance to have your say!

With an ever-increasing choice of holiday destinations and activities, young people with CF are more keen than ever to make the most of every opportunity for a more adventurous life. However, we encourage anyone planning a holiday to discuss their travel plans with the CF team.

Optimising health before travelling

Some patients may require a course of IV antibiotics before they travel to optimise their health. The CF team need to know travel dates in advance so that a plan for admission can be made. However, admission priority will always be for patients who are unwell and need treatment. Every effort is made to accommodate pre-holiday treatment but admission dates have to be flexible. Additionally, patients may need to consider taking back-up oral antibiotics with them.

There may be circumstances where patients are advised not to travel such as recent haemoptysis, recent pneumothorax, gastrointestinal obstruction or acute chest infections. These will be discussed with the consultants at the time. Unfortunately, travel plans may need to be postponed or cancelled.

Preparation

Fit-to-fly tests– If patients have an FEV1 of <50% or oxygen saturations of <95% they are advised to have a fitness-to-fly test, which will identify if oxygen is required on a flight. Patients should have a repeat test before each trip as their oxygen requirements may change.

Oxygen, nebulisers and compressors– Each airline has its own policy on oxygen transport and in-flight usage. Patients should contact their airline before they book in order to find out the policy. The airline will need to know whether oxygen is required during the entire flight or intermittently. Some airlines will provide oxygen free of charge or for a small fee. The airline will request a MEDIF form, which will need to be completed by a doctor from the CF team stating the patient’s oxygen requirements and that they are fit to travel.

Some low-cost airlines do not supply oxygen but they will allow patients to take their own approved oxygen supply on board (commonly, portable oxygen concentrators). Portable oxygen concentrators are fairly new but are increasingly being allowed on flights. Portable oxygen concentrators are lightweight and can run on electricity or battery. They are available to purchase but bear in mind they remain an expensive piece of equipment. If patients require oxygen at their destination, or during stop-overs, the patient should get in contact with their UK oxygen supplier who should be able to arrange this.

Medication– Patients are advised to pack a separate set of medication in their hand luggage as well as in their suitcase in case their luggage goes missing. If medicines need to be refrigerated, patients are advised to carry a cool bag or vacuum flask on board. Any equipment that will be needed during the flight should be battery powered. Most airlines will not allow the use of nebulisers during take-off or landing. If patients need to carry out treatment / medication during the flight they should inform the airline.

Power, pumps and plugs– Power supply varies from country to country. UK electrical appliances such as nebulisers may not work in certain countries. The physiotherapy department may be able to offer a travel compressor and patients should purchase electrical adapters for different plug sockets.

Paperwork– When travelling abroad patients should carry a letter from their CF team listing all the medicines and equipment that they will need to travel with (including needles, syringes, compressors). Travel insurance is essential and, though it may be more expensive, must cover “pre-existing” conditions or the patient will not be covered for CF-related treatment.

When travelling in the EU patients can apply for a European Health Insurance Card (EHIC). This entitles them to either free treatment or treatment at a reduced cost if they fall ill when travelling in Europe. It also covers treatment for chronic or pre-existing conditions. However, this should not be a substitute for travel insurance – it only covers emergency care and does not include some medication costs or repatriation.

Vaccinations and immunisations – Patients are advised to visit their GP as soon as possible to check if they need any vaccinations or other preventive measures (such as malaria tablets). Remember, these treatments are not usually available as NHS prescriptions.

Documentation required for patients to travel

Fitness-to-fly letter from doctor

A letter detailing current medical condition, medication and equipment

Travel insurance certificate

EHIC (if travelling in EU)

Places to avoid

Over recent years, there have been several cases of melioidosis. This is an infection caused by Burkholderia pseudomallei and can be potentially life threatening. Burkholderia pseudomallei is found in fresh water and damp soil in some areas of Asia and northern Australia.

Patients should also be aware of the potential risks of going to countries where hygiene standards and medical care are poor. Read the fact sheet from Australia's Northern Territory government and visit the CF Trust website for more information.

Risk of salt depletion

Heat can cause excessive sweating, which can lead to dehydration. Slo-Sodium (4-6 daily) is recommended. Patients travelling to hot countries and those who will be engaged in strenuous sports or other activities (e.g. cycling and skiing) must be prescribed enough for the duration of the holiday and are advised to stay well hydrated (particularly when drinking alcohol).

General travel health advice

Sometimes general health issues can be forgotten when planning travel. Therefore, patients are reminded to use a high-factor sunscreen and avoid excessive sunbathing, especially if they are on certain oral medication (e.g. voriconazole, ciprofloxacin). Patients are also reminded to practice safe sex and are advised to take condoms with them, as quality can vary in other countries.